41 Twenty years’ experience with juvenile dermatomyositis among Libyan children

Abstract Background Juvenile inflammatory myositis are systemic autoimmune diseases of unknown aetiology that are characterized by inflammation of skeleton, muscles, skin, and internal organs. Studies from different regions have reported different incidence and age at onset suggesting that the clinical and demographic features may differ by race and geographic regions. This study aims to describe the characteristics of disease among Libyan children who have been treated in the main rheumatology unit covering most of the population in Libya Objectives To describe the demographic and clinical features of patients with juvenile dermatomyositis To determine the outcome of children with juvenile dermatomyositis and factors affecting the outcome. Patients and methods This is a retrospective descriptive study conducted by reviewing patient records diagnosed with juvenile dermatomyositis from 5/2000–2/2022. Results Twenty-one patients were included, female to male ratio was 6:1. Mean age at disease onset was 7.7 ± 2.8 years and their mean follow-up period was 4.36 ± 3.2 years. Most of the patients (14.7%) presented before 1 month of starting symptoms. Most of the patients (13,0.9%), were diagnosed as dermatomyositis, 2 (9.5%) as polymyositis, 2 (9.5%) as amyopathic myositis, and 4 (19%) as overlap syndrome. Family History of dermatomyositis was positive in 4 (19%) of patients and family history of other autoimmune diseases was positive in 5 (23.8%) patients. Around half of the patients had monocyclic disease course (11, 52.4%), 5 patients (23.8%) had polycyclic disease course and other 5 patients (23.8%) had chronic persistent disease course. MRI proximal muscles and EMG were used for diagnosis in 10 patients (48%) and 16 (76.1%) respectively, and muscle biopsy was used in 2 patients to confirm the diagnosis. One overlap patient had severe lung fibrosis on CT scan chest with restrictive lung disease and 4 patients (19%) had restrictive lung disease with normal CT scan chest. Methyleprednisolone pulses were used in 10 patients (50%) with 3 (15%) who required more than one pulse of intravenous prednisolone. Immunoglobulin was used in 12 (60%) of the patients. All patients needed oral prednisolone and methotrexate was used in 15 (75%) of the patients. Other drugs used were Azathioprine in 3 overlap patients, cyclophosphamide and Mycophenolate mofetil in one patient with overlap syndrome. Hydroxychroroquine was used in 6 (30%) of the patients. In the last visit, 12 patients (60%) were in remission, 7 (58%) of them presented before 1 month of disease onset. Fifty-eight percent of the females and 66.7% of the males were in remission. Five patients (25%) had short stature, 3 (15%) had chronic cutaneous changes, 1 (5%) had arrythmia, 2 (10%) had calcinosis, 2 (10%) had osteoporosis, and one patient died. Conclusion A female predominance was noted with age at presentation comparable to other studies. The most frequent manifestations were skin manifestations and musculoskeletal features were the second most frequent symptoms. Patients has low rate of complications with low rate of calcinosis and mortality and none of them had gastrointestinal manifestations. Males were more likely to be in remission. 41 Table 1 summarizes the main clinical features at presentation: Feature Number and percentage Skin changes 17 (81%) Facial oedema 1 (4.8%) Hands and feet oedema 1 (4.8%) Fever 4 (19%) Generalized fatigability 12 (57.1%) Arthritis 11 (52.4%) Arthralgia 1 (4.8%) Muscle weakness 15 (71%) Dysphonia 4 (19%) Contractures 2 (9.5%)


Background
Oligoarticular type of juvenile idiopathic arthritis (oligoJIA) is considered the most common type of JIA. It usually affects one or < 4 joints. The knee is the most common affected joint followed by the ankle and wrist. Oligoarticular JIA presenting with erosive elbow monoarthritis as an initial sign of the disease is a rare condition which needs prompt diagnosis and management. Objective To recognize that isolated chronic elbow monoarthrits is an atypical presentation for oligoJIA. Case presentation: A 14-year-old volleyball player female child presented with chronic recurrent left elbow swelling of six months' duration. No history of morning stiffness, constitutional symptoms, other joint swelling, uveitis, or skin manifestations were reported. On examination there was mild cubitus valgus with elbow effusion associated with limited last degree elbow extension with no other signs of inflammation or infection (redness, tenderness, hotness). Hypermobility was noted in both elbows and knees. Patient was advised to rest the elbow and symptomatic treatment was recommended. On follow up there was mild improvement of the swelling, so further investigations were ordered. Erythrocyte Sedimentation Rate (ESR), C-reactive protein (CRP), Rheumatoid factor (RF) and antinuclear antibody (ANA) showed average normal values. X-ray of the elbow showed degenerative changes. Musculoskeletal ultrasound showed mild elbow effusion and magnetic resonance imaging (MRI) was ordered revealing effusion and early joint erosion at the ulnar articular surface with no synovial hypertrophy. Arthroscopy was recommended to detect the underlying pathology and histopathology revealed chronic active synovitis suggesting JIA. Non-steroidal antiinflammatory drugs (NSAIDs) were recommended but one month later knee arthritis was noted, so methotrexate was started and the condition started to improve.

Results
We report a 14-year-old female child who presented with atypical isolated chronic swelling of the elbow and was diagnosed by arthroscopy and histopathology. Non-steroidal anti-inflammatory drugs (NSAIDS) were tried as a first line treatment followed by methotrexate.

Conclusion
Chronic elbow monoarthritis is an atypical onset of oligoJIA and its diagnosis is important to prevent irreversible joint damage. Synovial biopsy can have a major role in the diagnosis of atypical monoarticular JIA and can help in early diagnosis and management improving the outcome. The implication to policy, practice, research, and advocacy: Monoarthritis of the elbow as initial sign of oligoJIA is an atypical onset of this disease and in the absence of systemic signs or other clinical indicators determining the exact cause of monoarthritis in children might be difficult. Synovial biopsy can have an important role in the diagnosis of monarticular arthritis in pediatric patients.

Background
In a large cohort of 2125 children followed in rheumatology, 2% had celiac disease (CD) (about 3 times the rate in the general population). Inversely, CD was reported in up to 2-3% of children with juvenile idiopathic arthritis Objectives Reporting joint damage such as arthrlagia in a celiac pediatric cohort Material and method We collected 58 celiac children and reported those with arthralgia and studied their clinical aspects Results Arthralgia was reported in 10/58 children (i.e. 17.24%) of the entire cohort studied (n ¼ 58), including 09 children with positive HLA DQ2/ DQ8 typing, potentially with an autoimmune background. The arthralgia was not accompanied by arthritis, and had an apyretic, labile and transient character. Only one case of episodic spinal pain was reported in the history Conclusion More than a sixth of the pediatric celiac cohort presents with arthralgia. These joint pains can be caused by vitamin D deficiency, osteoporosis, associated juvenile idiopathic arthritis (JIA), in particular HIV-positive. They can also be isolated and respond to the gluten-free diet.
Abstract citation ID: keac496.037 41 TWENTY YEARS' EXPERIENCE WITH JUVENILE DERMATOMYOSITIS AMONG LIBYAN CHILDREN Soad Hashad 1,2 , Hala Etayari 1,2 , Awatif Abushhaiwia 1,2 , Majeda Eltofil 1 , Aya Twati 1 , Zuhaira Awhidah 1 , Aeshah Ateeq 1 and Yusra Elfawires 2 1 Tripoli Children's Hospital, 2 The University of Tripoli Background Juvenile inflammatory myositis are systemic autoimmune diseases of unknown aetiology that are characterized by inflammation of skeleton, muscles, skin, and internal organs. Studies from different regions have reported different incidence and age at onset suggesting that the clinical and demographic features may differ by race and geographic regions. This study aims to describe the characteristics of disease among Libyan children who have been treated in the main rheumatology unit covering most of the population in Libya Objectives To describe the demographic and clinical features of patients with juvenile dermatomyositis To determine the outcome of children with juvenile dermatomyositis and factors affecting the outcome.

Patients and methods
This is a retrospective descriptive study conducted by reviewing patient records diagnosed with juvenile dermatomyositis from 5/2000-2/2022.

Results
Twenty-one patients were included, female to male ratio was 6:1. Mean age at disease onset was 7.7 AE 2.8 years and their mean followup period was 4.36 AE 3.2 years. Most of the patients (14.7%) presented before 1 month of starting symptoms. Most of the patients (13,0.9%), were diagnosed as dermatomyositis, 2 (9.5%) as polymyositis, 2 (9.5%) as amyopathic myositis, and 4 (19%) as overlap syndrome. Family History of dermatomyositis was positive in 4 (19%) of patients and family history of other autoimmune diseases was positive in 5 (23.8%) patients.
Around half of the patients had monocyclic disease course (11, 52.4%), 5 patients (23.8%) had polycyclic disease course and other 5 patients (23.8%) had chronic persistent disease course. MRI proximal muscles and EMG were used for diagnosis in 10 patients (48%) and 16 (76.1%) respectively, and muscle biopsy was used in 2 patients to confirm the diagnosis. One overlap patient had severe lung fibrosis on CT scan chest with restrictive lung disease and 4 patients (19%) had restrictive lung disease with normal CT scan chest. Methyleprednisolone pulses were used in 10 patients (50%) with 3 (15%) who required more than one pulse of intravenous prednisolone. Immunoglobulin was used in 12 (60%) of the patients. All patients needed oral prednisolone and methotrexate was used in 15 (75%) of the patients. Other drugs used were Azathioprine in 3 overlap patients, cyclophosphamide and Mycophenolate mofetil in one patient with overlap syndrome. Hydroxychroroquine was used in 6 (30%) of the patients. In the last visit, 12 patients (60%) were in remission, 7 (58%) of them presented before 1 month of disease onset. Fifty-eight percent of the females and 66.7% of the males were in remission. Five patients (25%) had short stature, 3 (15%) had chronic cutaneous changes, 1 (5%) had arrythmia, 2 (10%) had calcinosis, 2 (10%) had osteoporosis, and one patient died. Conclusion A female predominance was noted with age at presentation comparable to other studies. The most frequent manifestations were skin manifestations and musculoskeletal features were the second most frequent symptoms. Patients has low rate of complications with low rate of calcinosis and mortality and none of them had gastrointestinal manifestations. Males were more likely to be in remission. 41